Dejerine Sottas

Introduction to Dejerine Sottas

Dejerine-sottas syndrome is a condition affecting the peripheral nervous system (nerves which exit outside the spinal cord). It is a subtype of Charcot-Marie-Tooth (CMT) disease and is also known as hereditary motor sensory neuropathy type III. Its classification and name are still subject to debate. It differs from CMT disease in that its onset is typically during early infancy and is characterised by severe weakness and faster progression.

In Dejerine-Sottas syndrome the body attacks the protective coating of the nerves in the body which affects the transmission of nerve impulses and results in weakness and altered sensation. Diagnosis is usually made by a neurologist who may use additional investigations and tests such electromyography and nerve conduction studies to confirm the diagnosis.

Physiotherapy can be helpful in helping people with Dejerine-Sottas syndrome to maximise their independence for as long as possible.